A failing right heart in an eggshell: A case report of idiopathic constrictive pericarditis

Abstract

key Clinical Message

Constrictive Pericarditis is a rare fibrotic conversion of the pericardium that results in non‐specific clinical symptoms such as hepatomegaly, ascites, pleural effusions, and lower extremity edema. A multi‐modal diagnostic approach with cardiac imaging tools, cardiac hemodynamic measurements, and tissue biopsy can be used to diagnose Constrictive Pericarditis.

Abstract

Constrictive Pericarditis is a rare complication resulting in the fibrotic conversion of the pericardium secondary to idiopathic, infective, post‐surgical, or post‐radiation etiologies. The rigid and restrictive nature of the pericardium can result in non‐specific symptoms of volume overload that can mimic liver cirrhosis or congestive heart failure. We present the case of a 73‐year‐old female with constrictive pericarditis who presented with vague symptoms of abdominal pain, abdominal bloating, and bilateral lower extremity edema. This case report highlights the clinical manifestation, invasive, and non‐invasive diagnostic work‐up, and management of constrictive pericarditis.

1. INTRODUCTION

Constrictive Pericarditis (CP) is a rare fibrotic condition of the pericardium that is prevalent in approximately 9–10 cases per million individuals in the United States. ¹ CP presents as a rigid, noncompliant, thick, fibrotic, and/or calcification of the pericardium. ² On the contrary, a normal, healthy pericardial layer presents with fibroelastic tissue with a small amount of fluid. ³ CP can develop from infective causes, post‐cardiac surgery, or post‐mediastinal radiation. ¹ The progression of viral or idiopathic cases to CP is <1%, immune‐mediated or neoplastic CP is seen in 2%–5% of CP cases, and bacterial CP is seen in 20%–30% of CP cases. ³ Additionally, CP can present in 0.2%–0.4% of individuals post‐cardiac surgery. ⁴ CP presents with symptoms similar to those of restrictive cardiomyopathy, such as decompensated heart failure due to impaired ventricular filling. ¹ Additionally, CP can present with hepatomegaly and ascites secondary to hepatic congestion from congestive heart failure. ² We present a case of constrictive pericarditis in an elderly female with no significant medical history as an etiology for decompensated heart failure.

2. CASE HISTORY

The patient is a 73‐year‐old female with no significant medical history who presented to the Emergency Department (ED) with complaints of abdominal pain, abdominal bloating, and bilateral lower extremity edema of 2 weeks duration. Of note, she also mentioned intermittent chest pain of 1‐year duration that was sharp and substernal in nature with radiation to her back. Her social history was significant for moving from the Democratic Republic of the Congo 5 years prior to arrival. She denied nausea, vomiting, diarrhea, fevers, or chills. In the ED, her vital signs noted a heart rate of 77 beats/min, temperature of 98.8 oF, respiratory rate of 16 respirations/min, oxygen saturation of 94% on room air, and blood pressure of 112/88 mmHg. Her laboratory values are listed in Table 1.

TABLE 1.

Laboratory values in the emergency department.

Laboratory values	Patient's values	Normal range
N‐terminal pro‐brain natriuretic peptide (NT‐proBNP)	2425.9 pg/mL	<900 pg/mL
High sensitivity troponin (HST)	11 ng/L	<45 ng/L
Thyroid stimulating hormone (TSH)	0.534 uIU/L	0.340–4.82 uIU/L

3. METHODS

The chest radiograph shown in Figure 1 noted central pulmonary vascular congestion with bibasilar atelectasis, small to moderate right‐sided pleural effusion, small left pleural effusion, and mildly enlarged cardiac silhouette. Computed tomography (CT) of the abdomen and pelvis without contrast shown in Figure 2A,B noted large volume ascites, small bilateral pleural effusions, and small to moderate pericardial effusion with pericardial thickening. The electrocardiogram (EKG) shown in Figure 3 noted atrial fibrillation and diffuse T wave inversions at a rate of 82 beats/min. She was subsequently started on heparin drip and received intravenous furosemide.

FIGURE 1.

Chest radiography. Central pulmonary vascular congestion with bibasilar atelectasis, small to moderate right‐sided pleural effusion, small left pleural effusion, and mildly enlarged cardiac silhouette.

FIGURE 2.

(A, B) Computed tomography of the abdomen and pelvis without contrast. Large volume ascites noted by the yellow arrow, small bilateral pleural effusions noted by the black arrows, and small to moderate pericardial effusion with pericardial thickening noted by the red arrow.

FIGURE 3.

Electrocardiogram. Atrial fibrillation and diffuse T wave inversions at a ventricular rate 82 beats/min.

Transthoracic echocardiogram (TTE) shown in Figure 4A,B, and Video 1 noted left ventricular ejection fraction of approximately 50%–55%, estimated right ventricular systolic pressure of 45 mmHg, moderate mitral regurgitation, moderate to severe pulmonic regurgitation, small pericardial effusion, left pleural effusion, septal bounce and evidence of annulus reversus. She then had paracentesis with 1.8 L of serosanguinous fluid removed. Ascitic fluid showed reactive mesothelial cells and tested negative for malignancy. Due to evidence of annulus reversus on TTE, Cardiac Magnetic resonance imaging (MRI) shown in Figure 5A,B noted a calculated left ventricular ejection fraction of 56%, constrictive pericarditis with diffuse thickening of the pericardium, pericardial thickness greater than 4 mm in certain locations, late enhancement of the lateral pericardium, complex and moderate circumferential pericardial effusion, and right ventricular systolic function with calculated ejection fraction 51%. The patient underwent simultaneous Left and right heart catheterization to confirm the suspected diagnosis of constrictive pericarditis. Left heart catheteri zation showed evidence of normal epicardial coronary arteries without evidence of coronary atherosclerotic disease, and simultaneous coronary pressure waveform measure noted biventricular discordance with inspiration. Figure 6 depicts the hemodynamics of this patient consistent with constrictive pericarditis and evidence of elevated left and right sided filing pressure. The cardiac catheterization pressures are reported in Table 2.

FIGURE 4.

(A, B) Transthoracic echocardiogram. Annulus reversus displayed by the echocardiogram images with an elevated medial mitral annular velocity at 12.0 cm/s compared to the lateral mitral annular velocity at 10.9 cm/s.

VIDEO 1.

Transthoracic echocardiogram Ventricular interdependence indicated by septal bouncing as noted by the paradoxical bouncing of the interventricular septum during early diastole, moving towards then away from the left ventricle.

FIGURE 5.

(A, B) Cardiac magnetic resonance imaging. Cardiac MRI showing constrictive pericarditis with diffuse thickening of the pericardium highlighted by the white arrows.

FIGURE 6.

Hemodynamics of constrictive pericarditis in this patient. Hemodynamics displaying ventricular discordance during inspiration by an increase in right ventricular pressure indicated by the yellow arrow and a simultaneous decrease in left ventricular pressure indicated by the pink arrow. Early diastolic ventricular filling that is unimpeded and rapid followed by a late diastolic filling that halts abruptly when the total volume expands to the limit set by the stiff pericardium. Waveform of pressure measurements showing the square root sign displayed in the red box.

TABLE 2.

Right heart catheterization values.

Right heart catheterization values	Patient's values	Normal values
Right arterial pressure	17 mmHg	<5 mmHg
Right ventricular systolic pressure	49 mmHg	<25 mmHg
Right ventricular diastolic pressure	16 mmHg	<5 mmHg
Pulmonary artery systolic pressure	49 mmHg	<25 mmHg
Pulmonary artery diastolic pressure	24 mmHg	10 mmHg
Pulmonary capillary wedge pressure	23 mmHg	<12 mmHg
Left ventricular systolic pressure	122 mmHg	<130 mmHg
Left ventricular diastolic pressure	22 mmHg	10 mmHg
Aortic systolic pressure	117 mmHg	<130 mmHg
Aortic diastolic pressure	80 mmHg	90 mmHg
Cardiac index	2.86 L/min/m2	2.5–4 L/min/m2
Systemic vascular resistance	1340 d/s/cm5	770–1500 d/s/cm5
Pulmonary vascular resistance	132 d/s/cm5	20–120 d/s/cm5

4. CONCLUSION AND RESULTS

Patient denied any exposure to tuberculosis (TB) infected individuals and denied any classic symptoms of TB such as cough, fever, night sweats, and weight loss. However, due to lack of prior history of pericardiectomies, radiation therapy to her chest, or recurrent viral pericarditis, and recent travel from Congo, sputum acid‐fast bacillus and mycobacterium polymerase chain reaction (PCR) was tested to rule out TB. The patient tested negative for acid‐fast bacillus x3 and TB PCR, ruling out infectious TB as a cause for her constrictive pericarditis. Subsequently, she had a sternotomy and radical anterior and posterior pericardiectomy. Biopsy results from the pericardiectomy noted benign dense fibroconnective and adipose tissue with fibrosis and minimal chronic inflammation without acute inflammation. The pericardial tissue also tested negative for the Congo red stain ruling out amyloid deposition. The etiology for her constrictive pericarditis was deemed idiopathic as no cause was found.

The patient tested negative for acid‐fast bacillus x3 and TB PCR, ruling out infectious TB as a cause for her constrictive pericarditis. Subsequently, she had a sternotomy and radical anterior and posterior pericardiectomy. Biopsy results from the pericardiectomy noted benign dense fibroconnective and adipose tissue with fibrosis and minimal chronic inflammation without acute inflammation. The pericardial tissue also tested negative for the Congo red stain ruling out amyloid deposition. Due to persistent right sided pleural effusion, she then had pigtail chest tube placed. Additionally, the patient's diuresis was escalated to Bumex due to elevated volume status. Repeat TTE showed moderate and localized pericardial effusion around the left vertical with estimated left ventricular ejection fraction 55%–60%, no mitral regurgitation, and mild pulmonic regurgitation, and no doppler findings of pulmonary hypertension.

5. DISCUSSION

The clinical presentation of CP includes symptoms of fluid overload and/or reduced cardiac output/impaired ventricular filling such as jugular venous distention (JVD), pleural effusions, lower extremity edema, ascites, pulsus paradoxicus, and pulsatile liver. ² , ⁸ , ⁹ Patients present with the Kussmaul's sign due to JVD that persists during inspiration. ³ 80% of patients with CP present with JVD, 75% with peripheral edema, 40% present with ascites, and 60% with hepatomegaly. ² The stiff, restrictive pericardium reduces venous return into the ventricles during diastole resulting in an initial high driving pressure that abruptly drops when the pericardium reaches its elastic limit, causing a restrictive diastolic filling. ³ Owing to the vague and multiple symptoms of CP, it is a very difficult disease to diagnose. And can commonly be misdiagnosed as heart failure with preserved ejection fraction (HFpEF). ⁶ , ⁷ Due to the symptoms of ascites, pleural effusions, and lower extremity edema, CP may also be misdiagnosed as liver cirrhosis. ⁹

While there is no single pathognomic finding of CP, TTE is the initial diagnostic test that can depict right and left atrial dilation, dilated non‐collapsing inferior vena cava, thickened pericardium, diastolic septal bounce, respirophasic septal shift which is the displacement of the interventricular septum towards the left ventricle during inspiration. Multidetector cardiac computed tomography (MDCT) can be used to accompany the TTE to detect the location and extent of pericardial calcification. Additionally, cardiac MRI is highly sensitive and can be used to detect pericardial thickening seen in CP. ² Hemodynamic measurements can also be used to distinguish restrictive and constructive pericarditis. ² , ⁵ In CP, there is a significant reduction in the inflow across the mitral or bicuspid valve during expiration when compared to inspiration. A trans‐mitral flow variation of greater than or equal to 25% during respiration supports a diagnosis of CP. ²

Additionally, when considering CP, restrictive cardiomyopathy must also be included in the differential diagnosis. The brain naturetic peptide (BNP) is near normal, between 0 and 125 pg/mL, in constrictive disease but grossly elevated in restrictive disease. However, ultimately, an endomyocardial biopsy may be necessary to diagnose restrictive versus constrictive cardiomyopathy. ⁵ While not diagnostic, patients with CP endorse pericardial thickening of more than 3 mm. ⁶

CP is managed by surgical pericardiectomy but is associated with mortality rates of 11%–16% and increases with the New York Heart Association class. ⁸ Additionally, the hemodynamics post‐surgery can range from no recovery to complete recovery. The results may vary due to the surgical approach or from complete/incomplete pericardial resection. ⁶ Partial pericardiectomy is associated with 4.5 times greater risk of mortality post‐surgery; hence, total surgical pericardiectomy is advised for patients with class II or III impairment in the New York Heart Association classification. ⁸ Additionally, preoperative clinical status including age and co‐morbidities can impact the patient's prognosis. Patients of older age and with co‐morbidities such as pulmonary hypertension, multi‐organ failure, atrial fibrillation, or high mitral inflow tend to have poor prognosis post‐surgical therapy. Of note, patients with long standing CP may have “low‐output syndrome” during the early post‐operative period due to ventricular remodeling of the weakened myocardium. ⁹

However, patients may present with CP that is transient or reversible in nature, that can be managed by medical therapy. For instance, in CP secondary to viral infection or acute inflammation, non‐steroidal anti‐inflammatory drugs may resolve without surgical intervention. Follow‐up studies with imaging can be used to assess the patient. These patients may benefit from diuretics to manage their systemic congestion.

Owing to the non‐specific clinical manifestations of CP, it can be a very challenging disease to diagnose. Hence, a thorough and detailed history taking along with clinical suspicion is warranted to distinguish between CP mimickers such as Heart Failure preserved Ejection Fraction and liver cirrhosis. Additionally, a multi‐modal diagnostic approach with cardiac imaging tools such as MRI or MDCT along with cardiac hemodynamic measurements and tissue biopsy can confirm the diagnosis.

AUTHOR CONTRIBUTIONS

Nivedha Balaji: Conceptualization; formal analysis; investigation; methodology; resources; supervision; validation; visualization; writing – original draft; writing – review and editing. Sheriff N. Dodoo: Conceptualization; investigation; supervision; visualization; writing – review and editing. Alex Adams: Conceptualization; investigation; supervision; validation; writing – review and editing. Priyadarshini Dixit: Investigation; supervision; writing – review and editing. Giancarlo Acosta: Investigation; supervision; writing – review and editing. Jaime Burkle: Investigation; supervision; writing – review and editing.

FUNDING INFORMATION

No financial funding provided.

CONFLICT OF INTEREST STATEMENT

The author(s) declare(s) that there is no conflict of interest regarding the publication of this paper and the authors have no relevant disclosure.

ETHICS STATEMENT

Our institution does not require ethical approval to report individual cases.

CONSENT

Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.

Balaji N, Dodoo SN, Adams A, Dixit P, Acosta G, Burkle J. A failing right heart in an eggshell: A case report of idiopathic constrictive pericarditis. Clin Case Rep. 2024;12:e9277. doi: 10.1002/ccr3.9277

DATA AVAILABILITY STATEMENT

Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.